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Bicuspid aortic valve with left-noncoronary cusp fusion

MedGen UID:
1384871
•Concept ID:
C4476981
•
Congenital Abnormality
HPO: HP:0031121

Definition

A type of bicuspid aortic valve (BAV) characterized by a single raphe between the left and noncoronary cusps (LN fusion pattern). There is thus one completely developed right cusp, two completely developed commissures, and one raphe between the underdeveloped left and noncoronary coronary cusps extending to the corresponding malformed commissure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBicuspid aortic valve with left-noncoronary cusp fusion

Recent clinical studies

Etiology

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life.
Niaz T, Johnson JN, Cetta F, Poterucha JT, Hagler DJ
Mayo Clin Proc 2021 Jul;96(7):1874-1887. doi: 10.1016/j.mayocp.2020.12.033. PMID: 34218860
Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.
Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ
Congenit Heart Dis 2017 May;12(3):261-269. Epub 2016 Nov 28 doi: 10.1111/chd.12429. PMID: 27893194
Aortic dilatation with bicuspid aortic valves: cusp fusion correlates to matrix metalloproteinases and inhibitors.
Ikonomidis JS, Ruddy JM, Benton SM Jr, Arroyo J, Brinsa TA, Stroud RE, Zeeshan A, Bavaria JE, Gorman JH 3rd, Gorman RC, Spinale FG, Jones JA
Ann Thorac Surg 2012 Feb;93(2):457-63. Epub 2011 Dec 28 doi: 10.1016/j.athoracsur.2011.09.057. PMID: 22206960Free PMC Article

Diagnosis

Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life.
Niaz T, Johnson JN, Cetta F, Poterucha JT, Hagler DJ
Mayo Clin Proc 2021 Jul;96(7):1874-1887. doi: 10.1016/j.mayocp.2020.12.033. PMID: 34218860
Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.
Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ
Congenit Heart Dis 2017 May;12(3):261-269. Epub 2016 Nov 28 doi: 10.1111/chd.12429. PMID: 27893194

Prognosis

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434

Clinical prediction guides

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life.
Niaz T, Johnson JN, Cetta F, Poterucha JT, Hagler DJ
Mayo Clin Proc 2021 Jul;96(7):1874-1887. doi: 10.1016/j.mayocp.2020.12.033. PMID: 34218860
Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.
Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ
Congenit Heart Dis 2017 May;12(3):261-269. Epub 2016 Nov 28 doi: 10.1111/chd.12429. PMID: 27893194
Aortic dilatation with bicuspid aortic valves: cusp fusion correlates to matrix metalloproteinases and inhibitors.
Ikonomidis JS, Ruddy JM, Benton SM Jr, Arroyo J, Brinsa TA, Stroud RE, Zeeshan A, Bavaria JE, Gorman JH 3rd, Gorman RC, Spinale FG, Jones JA
Ann Thorac Surg 2012 Feb;93(2):457-63. Epub 2011 Dec 28 doi: 10.1016/j.athoracsur.2011.09.057. PMID: 22206960Free PMC Article

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